TY - JOUR
T1 - Identification and treatment of heterozygous familial hypercholesterolemia in children and adolescents
AU - Kwiterovich, Peter O.
N1 - Funding Information:
From the Lipid Research Atherosclerosis Unit, Departments of Pediatrics and Medicine, The Johns Hopkins University, School of Medicine, Baltimore, Maryland 21287-3654. This research was supported in part by a National Institutes of Health grants 1 P.50 HL47212-01 (Specialized Center of Research in Arteriosclerosis), 5UOl HL37975-06 (Dietary Intervention Study in Children), and General Clinical Research Center Program, RR-52, RR-35, and CLINFO.
PY - 1993/9/30
Y1 - 1993/9/30
N2 - Heterozygous familial hypercholesterolemia (FH) is completely expressed at birth and early in childhood by significant elevations in plasma total and tow density lipoprotein (LDL) cholesterol levels. High density lipoprotein cholesterol can be low in such FH children; the triglyceride levels are usually within the normal range. Screening of children for heterozygous FH using a LDL cholesterol level is reasonably efficient in families with known FH, but for general population screening, the LDL cholesterol level is often too nonspecific. Screening of offspring with a positive family history of premature coronary artery disease will provide a panoply of different lipoprotein phenotypes, reflecting the presence of other genetic conditions, including familial combined hyperlipidemia. Guidelines have been developed by the National Cholesterol Education Program (NCEP) Expert Panel on Blood Cholesterol levels in Children and Adolescents to assist in the evaluation and treatment of children with high LDL cholesterol levels. Although heterozygous FH probably counts for ≤5% of premature coronary artery disease, its identification and treatment are important, because FH often causes marked premature coronary artery disease early in adulthood, and can be successfully treated with a combined dietary and drug approach.
AB - Heterozygous familial hypercholesterolemia (FH) is completely expressed at birth and early in childhood by significant elevations in plasma total and tow density lipoprotein (LDL) cholesterol levels. High density lipoprotein cholesterol can be low in such FH children; the triglyceride levels are usually within the normal range. Screening of children for heterozygous FH using a LDL cholesterol level is reasonably efficient in families with known FH, but for general population screening, the LDL cholesterol level is often too nonspecific. Screening of offspring with a positive family history of premature coronary artery disease will provide a panoply of different lipoprotein phenotypes, reflecting the presence of other genetic conditions, including familial combined hyperlipidemia. Guidelines have been developed by the National Cholesterol Education Program (NCEP) Expert Panel on Blood Cholesterol levels in Children and Adolescents to assist in the evaluation and treatment of children with high LDL cholesterol levels. Although heterozygous FH probably counts for ≤5% of premature coronary artery disease, its identification and treatment are important, because FH often causes marked premature coronary artery disease early in adulthood, and can be successfully treated with a combined dietary and drug approach.
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U2 - 10.1016/0002-9149(93)90008-Z
DO - 10.1016/0002-9149(93)90008-Z
M3 - Article
C2 - 8213494
AN - SCOPUS:0027372798
VL - 72
SP - D30-D37
JO - American Journal of Cardiology
JF - American Journal of Cardiology
SN - 0002-9149
IS - 10
ER -